Atlas One · Host Biology · Scale 07 — System

Gorlin Syndrome

Gorlin syndrome (NBCCS) is caused by germline PTCH1 (~85%) or SUFU (~2%) mutations; multiple BCCs, odontogenic keratocysts, calcified falx cerebri, medulloblastoma (~5%); radiation avoidance is critical; vismodegib FDA-approved to reduce BCC burden in Gorlin patients.

Also known as: Gorlin syndrome, NBCCS, nevoid basal cell carcinoma syndrome, Basal Cell Nevus Syndrome, PTCH1 Gorlin, Gorlin-Goltz syndrome, Gorlin medulloblastoma, hereditary BCC, Gorlin PTCH1 SUFU

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
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gorlin-syndrome

Gorlin Syndrome

Gorlin syndrome (NBCCS) is caused by germline PTCH1 (~85%) or SUFU (~2%) mutations; multiple BCCs, odontogenic keratocysts, calcified falx cerebri, medulloblastoma (~5%); radiation avoidance is critical; vismodegib FDA-approved to reduce BCC burden in Gorlin patients.

Entry Metadata

FieldValue
IDgorlin-syndrome
NameGorlin Syndrome
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toSufu connects-toPtch1 connects-toSmo connects-toBasal Cell Carcinoma connects-toMedulloblastoma connects-toVegf connects-toSkin connects-toFibroblast connects-toP53 connects-toRhabdomyosarcoma

Sources